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1.
Bol Asoc Med P R ; 104(3): 41-6, 2012.
Article in English | MEDLINE | ID: mdl-23156891

ABSTRACT

Chronic myeloid neoplasm with eosinophilia and abnormality of platelet-derived growth factor receptor alpha (PDGFRA), referred as chronic eosinophilic leukemia, is an extremely rare neoplasm where long-term prognosis is uncertain though a high grade of responsiveness to Imatinib has been reported. The mortality and morbidity associated with chronic eosinophilic leukemia is associated with the degree of tissue involvement, damage, or both at diagnosis. We discuss a case of a young male patient with past medical history of hypoglycemia that presented to the emergency room with a complaints of a sharp abdominal pain localized in the upper quadrants. Laboratories were remarkable for elevated white blood cells with eosinophils predominance, anemia and thrombocytopenia. Bone marrow biopsy dislocated a FIP1L1-PDGFRA fusion gene chronic eosinophilic leukemia. Physicians need to have a high index of suspicion of this rare entity since not all eosinophilias can be interpreted as asthma or parasitis infections.


Subject(s)
Hypereosinophilic Syndrome/complications , Leukemia, Myeloid/complications , Receptor, Platelet-Derived Growth Factor alpha , Asthma , Decision Trees , Humans , Male , Parasitic Diseases , Young Adult
2.
Biol Blood Marrow Transplant ; 15(6): 656-61, 2009 Jun.
Article in English | MEDLINE | ID: mdl-19450749

ABSTRACT

Treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) has been traditionally empirical, primarily aiming at ameliorating symptoms or treating complications resulting from the disease. Novel therapies such as eculizumab result in stabilization of hemoglobin levels and improvement in quality of life, but does not cure PNH. Nonrandomized studies suggest that long-term remissions are achievable when using myeloablative or nonmyeloablative/reduced-intensity (NMT/RIC) allogeneic hematopoietic stem cell transplantation (HSCT) as treatment for PNH. Nevertheless, patients with previous life-threatening complications from PNH may be more appropriately treated with an NMT/RIC regimen, rather than a myeloablative approach, because of the increased transplant mortality associated with the latter. The decision to perform an allogeneic HSCT (allo-HSCT) should weigh disease prognosis, by incorporating known adverse prognostic factors such as previous history of thrombosis and/or evolution to pancytopenia, among others, against the risk of transplant-related complications. Selection of the appropriate candidate and, equally important, the right time to perform an allo-HCT are important questions that need to be answered in the context of large prospective randomized trials.


Subject(s)
Hematopoietic Stem Cell Transplantation , Hemoglobinuria, Paroxysmal/surgery , Adolescent , Adult , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized , Anticoagulants/therapeutic use , Blood Transfusion , CD55 Antigens/analysis , CD59 Antigens/analysis , Child , Clinical Trials as Topic/statistics & numerical data , Danazol/therapeutic use , Glucocorticoids/therapeutic use , Hematopoietic Stem Cell Transplantation/mortality , Hemoglobinuria, Paroxysmal/diagnosis , Hemoglobinuria, Paroxysmal/drug therapy , Hemoglobinuria, Paroxysmal/therapy , Humans , Middle Aged , Myeloablative Agonists/administration & dosage , Myeloablative Agonists/adverse effects , Postoperative Complications/prevention & control , Prognosis , Remission Induction , Risk Factors , Thrombophilia/etiology , Transplantation Conditioning/methods , Transplantation Conditioning/mortality , Transplantation, Homologous , Young Adult
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